A child of Italian parentage is presented as an example of Hgb H-thalassemia disease. A family study is included. Previous documented reports have included individuals of Chinese, Thai, Greek and Filipino parentage. Genetic aspects of the association of Hgb H with thalassemia are discussed. It is suggested that the study of other patients who present a clinical picture resembling mild Cooley’s anemia may reveal the presence of hemoglobin variants.